Saturday, 6 August 2016

Atypical AA3 in a 20 YO

Hello everyone:

I am grateful to have this group. My sister, 20, has been recently diagnosed with an anaplastic astrocytoma, IDH wildtype. Her ATRX and p53 proteins are intact, and she has the TERT promoter (pTERT) mutation.
Her tumor is quite unique, in the sense that it is not exactly an adult glioma nor a pediatric one. It has a mutation in the ARID2 gene, which is commonly found in liver cancer and is associated with tumor recurrence.
She has had subtotal resection of her large left insular tumor, and her medical team are now “wrestling” to find an appropriate treatment for her. I have a few questions, and I appreciate your input on them:
  1.   Notable labs: has anyone tried this start up? Its initial mission was to help brain cancer patients find FDA approved drugs that can control their disease but it seems to have changed its direction. It is running a clinical trial for leukemia patients.
  2. Survivorship: is anyone aware of a long-term survivor of anaplastic astrocytoma, IDH wild type? Some papers on Pubmed indicate that very few make it past two years following treatment.
  3. Depression: my sister is, quite understandably, depressed and resents the idea of having to undergo radiation and chemotherapy. I want to convince her of the benefits of the cocktail approach, but I have no idea how to open the subject to her. Is any of the caregivers here experienced in this matter?
Thank you all.


  1. It's true this tumor sounds quite unusual, not falling into any of the common categories of pediatric or adult gliomas. The main thing it has in common with adult GBM is the TERT promoter mutation. ARID2 mutation on the other hand is very rare in glioma. ARID1A and ARID1B mutations are somewhat more common, occurring in about 7% and 5% of low grade oligodendrogliomas.

    1) I'm very familiar with Notable. One thing that's important to know concerning Notable is that they would require fresh tissue shipped to them within 24 hours or so of surgery - they can't use preserved tissue for their assay (they can't use paraffin-embedded or flash frozen bulk tumor, it must be fresh).

    2. I wouldn't pay attention to statistics for IDH wild type AA, as most of these turn out to be identical to adult GBM from a genetic point of view (they tend to share the same common genetic and chromosomal alterations as primary GBM). Your sister's tumor doesn't seem to fall into that category, so any kind of informed attempt at a prognosis is impossible as far as I can tell. That said, I know of long term survivors of IDH wild type GBM, a slightly more serious diagnosis than IDH wild type AA, so there's always hope.

    As far as motivation, many of us here were inspired by Ben Williams story and book (Surviving Terminal Cancer). It provides living proof that going beyond standard treatments can lead to long-term survival. Watching parts of Dominic Hill's film Surviving Terminal Cancer, which goes into the stories of long-term survivors such as Ben, Rich Gerber, and Anders Ferry can also be inspiring. You may want to watch it yourself first before judging what you should expose her to and what she is ready for. She may not be accepting of the fact that the medical profession considers this to be an incurable disease. But I think exposing her to examples of long-term survivors who pursued non-standard but science-based treatments is a good start. See also my friend Jess's blog ( and Cheryl Broyles site (

    1. Stephen - thank you. I am familiar with the stories of Jessica and Cheryl and have even watched Dominic Hill's film but I was too afraid to make her watch it in its entirety. Your suggestion is brilliant, I will let her watch selected part of the film. Thanks again.

    2. Hello Meer,

      maybe visit The author is 20-30 year old male, diagnosed with anaplastic astrocytoma and successfully following ketogenic diet. Somewhere in between the posts I think he wrote that there was still a spot left after surgery, but now (3 years later) the spot was regressing. He also posts a lot of scientific papers regarding metabolic approach (mainly ketogenic diet)